A Case Report of Recurrent Guillain-Barré Syndrome with Orthostatic Hypotension Syncope as the First Symptom

Guillain⁃Barré syndrome (GBS) is an immune-mediated peripheral neuropathy with acute or subacute onset of flaccid paralysis of the limbs with symmetrical hypesthesia and autonomic nerve involvement [1]. The clinical manifestations of autonomic nerve damage are complex and varied, which may involve extensive or limited autonomic function damage, including abnormalities of the skin, pupil, urinary tract, gastrointestinal tract, cardiovascular system, body temperature, lacrimal and salivary glands, and sexual function, etc. [2], and some patients may even have autonomic nerve damage as the only symptom, which is a variant of GBS and is prone to misdiagnosis or underdiagnosis. Recurrence of GBS is rare, and the manifestations of recurrence are often similar to those of the first symptoms [3], but the patient admitted to our hospital had syncope as the main clinical manifestation of recurrence, which was completely different from that of the first incidence, and syncope is not a common and typical clinical manifestation of GBS, so misdiagnosis is highly likely.

Peroral endoscopic myotomy for treatment of Guillain-Barre syndrome-associated achalasia: A rare case

Guillain-Barre syndrome (GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type I achalasia who was successfully treated with peroral endoscopic myotomy (POEM). A 30-year-old man who was diagnosed with GBS 3 mo before was referred to our department with dysphagia and meal-related regurgitation. The results of esophagography, endoscopy, and high-resolution manometry (HRM) revealed type I achalasia. POEM that utilized a submucosal tunneling technique was performed to treat the GBS-associated type I achalasia. After POEM, smooth passage of a contrast agent into the stomach was shown in follow-up esophagography, and follow-up HRM revealed a decrease in the mean integrated relaxation pressure 22.9 mmHg to 9.6 mmHg. The patient remained without dysphagia for 7 mo, even though the patient’s neurological problems were not fully resolved. POEM may be a safe and effective treatment for GBS-associated type I achalasia.

A Case-control Study on Children with Guillain-barre Syndrome in North China

To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined by electrophysiology. Serum IgG antibodies specific for C. jejuni were determined in all the subjects by ELISA. Each case and control were interviewed using an ad hoc questionnaire, including his/her demographic information,onset of the illness, their personal hygiene and so on. Results The study showed that Guillain-barre syndrome was associated with a few factors, such as polio vaccine immunization before onset of illness (OR=7.27), no hand washing after defecation and before meals (OR=6.15). Infection of C. jejuni was strongly associated with the illness (OR=9.5,P<0.001). Conclusion It is suggested that occurrence of Guillain-barre syndrome may correlate to infection of C. jejuni and poor personal hygiene in children.

Effects of increased human tumor necrosis factor-like molecule 1A expression in peripheral blood of children with acute Guillain-Barre syndrome on interferon-gamma secretion

BACKGROUND： Human tumor necrosis factor-like molecule 1A （hTL1A） is a strong T helper cell type 1 （Thl） co-stimulator. Guillain-Barre syndrome （GBS） is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE： To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING： A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS： Venous blood samples were obtained from 6 healthy donors, aged 6-12 years （all routine blood examination items were normal）, and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A （rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form） was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS： Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups： control （2 μg/mL phytohemagglutinin）, 2μg/mL phytohemagglutinin ＋ 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine （3H-TdR） method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay （ELISA）. The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES： The following parameters were measured： rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS： T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group （P 〈 0.05）. The ratio of hTLIA＋ CD3＋ T cells to CD3＋ T cells in acute GBS children was significantly greater than the control group （P 〈 0.01 ）. Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased （P 〈 0.05）. CONCLUSION： In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.

Guillain-Barre syndrome associated with peginterferon alfa-2a for chronic hepatitis C: A case report

The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.

A rare presentation of Guillain-Barre syndrome with GQ1b positivity:A case report

Rationale:To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity.Patient concern:A 35-year-old female,68 days postpartum,presented with headache,vomiting,and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking,13 days after ChAdOx1 nCoV-19 vaccination.Diagnosis:Guillain-Barre syndrome with GQ1b positivity.Intervention:Five cycles of plasmapheresis were given.Outcome:The patient’s clinical condition improved.Palatal weakness improved and she could walk without support.There were mild sensory symptoms involving upper limbs which gradually improved.Lessons:AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination.Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.

Asymmetric limb weakness in Guillain-Barre syndrome:Three case reports

BACKGROUND Guillain-Barrésyndrome(GBS)is an autoimmune-mediated peripheral neuropathy characterized by symmetric weakness.Asymmetric weakness in GBS is uncommon and may be easily confused with other differential diagnoses.We herein present three cases of asymmetric GBS and review the literature on this atypical subtype of GBS in order to describe the characteristics of asymmetric GBS and to provide experience for clinicians.CASE SUMMARY Different from patients in the previous reports,our patients showed persistent asymmetric limb weakness from the onset to recovery phase.All three patients were serologically positive for antecedent infections.Two of the three cases had IgG antibodies against ganglioside GM1.Two patients received immunotherapy including intravenous immunoglobulin and plasma exchange,while one patient received only supportive treatment.Autoantibodies against gangliosides,asymmetry of congenital development of blood-nerve barrier and limb use may contribute to the development of asymmetric limb weakness in GBS.CONCLUSION Asymmetric GBS may be a rare clinical variant and should be considered when a patient develops acute and progressive asymmetric limb weakness.The differences in clinical features and prognosis between asymmetric GBS and classic GBS deserve further investigation in a large study.

Association of neuroelectrophysiology and analysis of cerebrospinal fluid immunoglobulin with pathogenetic conditions of patients with Guillain-Barre syndrome

BACKGROUND： Guillain-Barre syndrome （GBS） is an autoimmune disease which is characterized by demyelination of peripheral nerve and nerve root, and inflammatory reaction of lymphocyte and macrophage. Neuroelectrophysiological examination and cerebrospinal fluid （CSF） analysis are of significance for its diagnosis. OBJECTIVE： To study the association of neuroelectrophysiology and cerebrospinal fluid immunoglobulin （CSF-lg） with pathogenetic conditions of patients with GBS. DESIGN： Case control study SETTING： Department of Neurology, Shenzhen Municipal Shekou Group Hospital; Department of Neuroelectrophysiology, People＇s Hospital of Guangdong Province. PARTICIPANTS： A total of 32 GBS patients including 18 males and 14 females who aged from 17 to 72 years were selected as experimental group from the Department of Neurology, People＇s Hospital of Guang- dong Province from January 2004 to December 2005. All cases conformed with GBS diagnostic criteria established by Asbury in 1990 and they were divided into three types according to neurological criteria established by Chinese Neurology and Psychology Journal in 1993： mild, moderate and severe types. Another 30 patients with vascular headache were selected as control group from the same hospital including 14 males and 16 females who aged from 17 to 79 years. METHODS： ① Neuroelectrophysiological examination： Multiple-functional electromyography device provided by Nicolet Company, USA was used to measure nerve conduction velocity （NCV）, including motor nerve conduction velocity （MCV） and sensory nerve conduction velocity （SCV）; meanwhile, electromyologram （EMG）, somatosensory evoked potential （SEP） and electroencephalogram （EEG） were also measured. ② Detection of CSF-lg： Concentrations of IgG, IgA and IgM were measured with immunofixation electrophoresis. ③Follow-up： Among 32 GBS patients, 14 cases received follow-up after treatment and the longest fol- low-up time was 1 year after onset. Among them, 8 cases were reexaminined with neuroelectrophysiological and CSF examinations. MAIN OUTCOME MEASURES： Results of NCV, EMG, SEP and EEG; comparison of CSF-lg content; results of follow-up examinations. RESULTS： All 32 GBS cases and 30 patients with vascular headache were involved in the final analysis. ① Abnormal rate of neuroelectrophysiological test： 75% of NCV, 88% of F-wave, 53% of MCV, 25% of SEP, 47% of EMG and 31% of EEG. There were no significant differences among various types （P 〉 0.05）. ② Results of CSF-lg test： There were no significant differences among various types （P 〉 0.05）; however, abnormalities in experimental group was higher than those in control group （P 〈 0.01）. CONCLUSION ： Results of follow-up study suggest that improvement of clinical symptom is earlier than neuroelectrophysiological recovery; MCV and EMG recoveries are faster than that of NCV; the earlier the abnormality of EMG, the poorer the recovery. CSF4g recovers normally along improvement of clinical symptoms. It is of significance for neuroelectrophysiology and abnormality of CSF-Ig to determine degree of peripheral nerve demyelination and prognosis.

Clinically Diagnosed Guillain-Barre Syndrome in Pregnancy: Case Report and Review of Literature

Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dysfunction and respiratory failure in severe cases and ultimately death if not promptly diagnosed and treated. It may be preceded by an antecedent event in about two-third of cases. This could be an upper respiratory tract infection, viral illness, recent history of vaccination, pregnancy, cancer or even trauma. The condition is exceedingly rare in pregnancy and only few cases have been reported in literature. Case Report: This is a case of a 28-year-old Gravida 3, Para 1+1 and Estimated Gestational Age of 30 weeks and 4 days. There was a history of upper respiratory tract infection eight weeks prior to presentation which spontaneously resolved. On examination, she was a young woman, anxious, weak, afebrile, not pale, the neck could not hold the head upright and there was bilateral non tender pitting pedal oedema extending to her mid-shin. There were no cranial nerve deficits and no sign of meningeal irritation. There were normal muscle bulk with global hypotonia and flaccid quadriparesis, Power was 3/5. The proximal groups of muscles were more affected than the distal parts. Reflexes were diminished globally with plantar flexor response. She had immunoglobulin as treatment. Conclusion: In a low resource setting like ours it is important to have a high index of suspicion of GBS when an apparently healthy gravid woman presents with progressive weakness of the limbs.

Exceptional Association of a Cerebral Sinus Thrombosis and a Guillain-Barre Syndrome: A New Case Report and Review of the Literature

Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Guillain-Barre syndrome. He had symptomatic treatment in addition to plasma exchange. In the presence of clonic movements, a brain venography magnetic resonance showed a thrombophlebitis of the left lateral sinus, and hence a low-molecular-weight heparin treatment was begun. Immunological, thrombophilia and serological tests were negative. After a favorable evolution, he was transferred to the neurology department. Conclusion: The combination of a Guillain-Barre syndrome and a cerebral sinus thrombosis would suggest a common process. A rigorous investigation, including the use of imaging, is necessary in front of any unusual clinical sign during a GBS.

COVID-19 Infection Presenting as Myalgia, Abnormal Liver Function Tests and the Guillain-Barre Syndrome

The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavirus disease 2019 (COVID-19) with case reports suggesting an association with the Guillain-Barre syndrome. Most patients present with the typical upper respiratory symptoms in association with these neurological symptoms. We present a case of an unvaccinated gentleman with none of the typical respiratory symptoms of COVID-19 who presented with the Guillain Barre syndrome and myalgia. His symptoms settled following treatment with intravenous immunoglobulins. This case highlights the importance of testing for COVID-19 in patients without typical symptoms but who present with neurological illness and supports the use of intravenous immunoglobulin therapy.

Dynamic ultrasonography for optimizing treatment position in superior mesenteric artery syndrome:Two case reports and review of literature

BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome,individual variations in the optimal patient position have been noted.In this report,we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient.CASE SUMMARY Case 1:A 90-year-old man with nausea and vomiting.Following diagnosis of SMA syndrome by computed tomography(CT),ultrasonography(US)revealed the SMA-Ao distance in the supine position(4 mm),which slightly improved in the lateral position(5.7–7.0 mm)without the passage of duodenal contents.However,in the sitting position,the SMA-Ao distance was increased to 15 mm accompanied by improved content passage.Additionally,US indicated enhanced passage upon abdominal massage on the right side.By day 2,the patient could eat comfortably with the optimal position and massage.Case 2:An 87-year-old woman with vomiting.After the diagnosis of SMA syndrome and aspiration pneumonia by CT,dynamic US confirmed the optimal position(SMA-Ao distance was improved to 7 mm in forward-bent position,whereas it remained at 5 mm in the supine position).By day 7 when her pneumonia recovered,she could eat with the optimal position.CONCLUSION The optimal position for SMA syndrome varies among individuals.Dynamic US appears to be a valuable tool in improving patient outcomes.

Miller fisher syndrome with positive anti-GQ1b/GT1a antibodies associated with COVID-19 infection:A case report

BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial roles in the development of MFS.The positive rate of ganglioside antibodies is exceptionally high in MFS patients,particularly for anti-GQ1b antibodies.However,the presence of other ganglioside antibodies does not exclude MFS.CASE SUMMARY We present a 56-year-old female patient who suddenly developed right blepharoptosis and progressively worsening vision in both eyes.There were flu symptoms prior to onset,and a coronavirus disease 2019 test was positive.On physical examination,the patient exhibited bilateral extraocular muscle paralysis,weakened reflexes in both limbs,and impaired coordination.The cerebrospinal fluid examination results showed no obvious abnormalities.Bilateral peroneal nerve F-waves were not extracted.Serum anti-GD1b IgG and anti-GT1a IgG antibodies were positive.The patient received intravenous methylprednisolone(1000 mg/day),with the dosage gradually decreased.Additionally,intravenous high-dose immunoglobulin treatment was administered for 5 days(0.4 g/kg/day)from day 2 to day 6 of hospitalization.The patient’s symptoms improved after treatment with immunoglobulins and hormones.CONCLUSION Positive ganglioside antibodies may be used as supporting evidence for the diagnosis;however,the diagnosis of MFS is more reliant on clinical symptoms.

Nasogastric tube syndrome:A Meta-summary of case reports

BACKGROUND Since its description in 1790 by Hunter,the nasogastric tube(NGT)is commonly used in any healthcare setting for alleviating gastrointestinal symptoms or enteral feeding.However,the risks associated with its placement are often underes-timated.Upper airway obstruction with a NGT is an uncommon but potentially life-threatening complication.NGT syndrome is characterized by the presence of an NGT,throat pain and vocal cord(VC)paralysis,usually bilateral.It is poten-tially life–threatening,and early diagnosis is the key to the prevention of fatal upper airway obstruction.However,fewer cases may have been reported than might have occurred,primarily due to the clinicians'unawareness.The lack of specific signs and symptoms and the inability to prove temporal relation with NGT insertion has made diagnosing the syndrome quite challenging.AIM To review and collate the data from the published case reports and case series to understand the possible risk factors,early warning signs and symptoms for timely detection to prevent the manifestation of the complete syndrome with life-threatening airway obstruction.METHODS We conducted a systematic search for this meta-summary from the database of PubMed,EMBASE,Reference Citation Analysis(https://www.referencecitation-analysis.com/)and Google scholar,from all the past studies till August 2023.The search terms included major MESH terms"Nasogastric tube","Intubation,Gastrointestinal","Vocal Cord Paralysis",and“Syndrome”.All the case reports and case series were evaluated,and the data were extracted for patient demographics,clinical symptomatology,diagnostic and therapeutic interventions,clinical course and outcomes.A datasheet for evaluation was further prepared.RESULTS Twenty-seven cases,from five case series and 13 case reports,of NGT syndrome were retrieved from our search.There was male predominance(17,62.96%),and age at presentation ranged from 28 to 86 years.Ten patients had diabetes mellitus(37.04%),and nine were hypertensive(33.33%).Only three(11.11%)patients were reported to be immunocompromised.The median time for developing symptoms after NGT insertion was 14.5 d(interquartile range 6.25-33.75 d).The most commonly reported reason for NGT insertion was acute stroke(10,37.01%)and the most commonly reported symptoms were stridor or wheezing 17(62.96%).In 77.78%of cases,bilateral VC were affected.The only treatment instituted in most patients(77.78%)was removing the NG tube.Most patients(62.96%)required tracheostomy for airway protection.But 8 of the 23 survivors recovered within five weeks and could be decannulated.Three patients were reported to have died.CONCLUSION NGT syndrome is an uncommon clinical complication of a very common clinical procedure.However,an under-reporting is possible because of misdiagnosis or lack of awareness among clinicians.Patients in early stages and with mild symptoms may be missed.Further,high variability in the presentation timing after NGT insertion makes diagnosis challenging.Early diagnosis and prompt removal of NGT may suffice in most patients,but a significant proportion of patients presenting with respiratory compromise may require tracheostomy for airway protection.

Thiamine responsive megaloblastic Anemia and deafness: A rare case of Roger’s syndrome with successful hearing rehabilitation by cochlear implantation

Thiamine responsive megaloblastic anemia(TRMA),also known as Roger’s syndrome,is an exceptionally rare autosomal recessive disorder stemming from mutations in the SLC19A2 gene responsible for encoding a thiamine carrier protein.This syndrome manifests as the classic triad of megaloblastic anemia,sensorineural hearing loss,and diabetes mellitus.Here,we present the case of a one-and-a-half-year-old male infant born to non-consanguineous parents in India,a region where TRMA cases are seldom reported.At five months of age,the child exhibited the characteristic symptoms,prompting immediate treatment involving thiamine therapy,insulin administration,and blood transfusions.Notably,the child exhibited significant improvement in all aspects except for hearing loss,which conventional hearing aids failed to alleviate.However,following a cochlear implant procedure conducted within a few months,the child regained hearing abilities.This case underscores the importance of early recognition and intervention in the form of cochlear implant,demonstrating the potential to reverse TRMA symptoms and provide affected individuals with a substantially improved quality of life.

Ziwan-Taoren herb pair can exert an therapeutical effect in primary Sjogren’s syndrome through inhibiting the TLR/NF-κB pathway

Background:Ziwan and Taoren(ZT)is a classic medicine pair in the formula of Mai Dong Di Shao Decoction,has been used to treat primary Sjogren’s syndrome(pSS)for more than 20 years.But its action mechanism is still unknown.This study is aimed to reveal the potential mechanism of ZT treated pSS and discover its active compounds of ZT and therapeutic target for pSS.Methods:Firstly,the potential pathways of ZT for pSS treatment were predicted through network pharmacology and GO and KEGG enrichment analysis.Secondly,the inter-structural relationships between active compounds of ZT and target proteins were visualized using molecular docking techniques.Finally,efficacy and mechanism were conducted through in vivo experiments,such as water intake,spleen index,hematoxylin-eosin staining pathological changes,ELISA,Western Blot analysis,and immunofluorescence staining.Results:Nine active compounds were extracted from network pharmacology,including quercitrin,luteolin,kaempferol,β-sitosterol,isorhamnetin,galangin,hederagenin,diosmetin and gibberellin 7.Seven disease targets were identified:RELA,TP53,AKT1,interleukin(IL)6,MAPK1,ESR1,IL10;with RELA being the most core target.KEGG and GO enrichment analysis indicated that ZT may act through the TLR/NF-κB/RELA inflammatory mechanism process.preliminary results of molecular docking showed that ZT’s active compounds bind well to the RELA(p65)receptor.In vivo results demonstrated that a high dose of ZT significantly improved water intake and reduced lymphocytes infiltration in submandibular gland pathology in NOD mice.The expression content of AQP5 and vasoactive intestinal peptide in the submaxillary gland was significantly increased,while levels of inflammatory factors such as tumor necrosis factor-α,IL-6,and IL-1βalong with protein expressions including toll-like receptor4,p-p65 and p-IKKα/βin NF-κB pathway were reduced.Conclusions:The ZT treatment exhibits a promising efficacy in mitigating dryness symptoms of pSS,potentially attributed to its capacity for suppressing the TLR/NF-κB inflammatory signaling pathway.

Successful splenic artery embolization in a patient with Behçet’s syndrome-associated splenic rupture:A case report

BACKGROUND Splenic rupture associated with Behçet’s syndrome(BS)is extremely rare,and there is no consensus on its management.In this case report,a patient with BSassociated splenic rupture was successfully treated with splenic artery embolization(SAE)and had a good prognosis after the intervention.CASE SUMMARY The patient was admitted for pain in the left upper abdominal quadrant.He was diagnosed with splenic rupture.Multiple oral and genital aphthous ulcers were observed,and acne scars were found on his back.He had a 2-year history of BS diagnosis,with symptoms of oral and genital ulcers.At that time,he was treated with oral corticosteroids for 1 month,but the symptoms did not alleviate.He underwent SAE to treat the rupture.On the first day after SAE,the patient reported a complete resolution of abdominal pain and was discharged 5 d later.Three months after the intervention,a computed tomography examination showed that the splenic hematoma had formed a stable cystic effusion,suggesting a good prognosis.CONCLUSION SAE might be a good choice for BS-associated splenic rupture based on good surgical practice and material selection.

What Are the Current and Developing Treatments for Cotard’s Syndrome, Alice in Wonderland Syndrome, and Catatonic Schizophrenia?

Purpose: Cotard’s syndrome, Alice in Wonderland Syndrome, and Catatonia are all rare psychiatric disorders that have relatively little research regarding their treatments. The aim of this article is to highlight any gaps in knowledge regarding represented demographics in these treatment studies, and to discuss the current and upcoming treatment options. Background: This literature review explores under-researched psychiatric conditions: Cotard’s syndrome, Alice in Wonderland syndrome, and Catatonic Schizophrenia. Understanding psychiatric disorders requires basic knowledge of brain anatomy. These conditions are often result of or associated with neurological issues, such as migraines or tumors. The brain has eight lobes, two of four kinds: frontal, parietal, occipital, and temporal lobes, which all govern different functions and abilities. Frontal lobes control judgment, decision-making, personality traits, and fine motor movements. Parietal lobes interpret pain and temperature, occipital lobes handle visual stimuli, and temporal lobes enable hearing. The pre-frontal cortex is associated with high intelligence, psychotic traits, and psychosis. The Broca’s Area in the frontal lobes controls expressive language. These areas and divisions of the brain contribute to the complexity of the psychiatric disorders discussed in this review. Introduction: Cotard’s syndrome is a psychiatric disorder characterized by delusions of being dead or not having certain limbs or organs. It is believed that there is a disconnect between their fusiform face area and the amygdala, causing a lack of familiarity between one’s mind and body. Alice in Wonderland Syndrome (AIWS) is another psychiatric disorder which is characterized by visual hallucinations, such as distorted perceptions of color, size, distance, and speed. The most common symptoms include micropsia and macropsia. Catatonia/Catatonic Schizophrenia is an uncommon type of schizophrenia. This type of schizophrenia is characterized by motor rigidity, verbal rigidity, the flat effect, psychomotor retardation, waxy flexibility, and overall negative symptoms. Thus, these people may come off as emotionally detached, and able to stay frozen in odd positions for periods on end. Treatments and Results: Cotard’s syndrome seemed to be most effectively treated by ECT (electroconvulsive therapy). Alice in Wonderland Syndrome (AIWS) had the highest positive responses to treatment by Valproate (an anti-epileptic drug), as well as intervention to treat the associated neurological conditions they had. Catatonia/Catatonic Schizophrenia seemed to be most effectively treated with a combination of benzodiazepines and ECT. Discussion and Demographics: In all 3 disorders, the Latino and African communities were underrepresented. There also seemed to be an underrepresentation of men in Cotard’s syndrome, and of women in Alice in Wonderland Syndrome. Japan and India seemed to have the highest density of treatment studies in all 3 disorders.

A Typical Case of Classic Potter’s Syndrome: A Case Report

Potter syndrome is a rare congenital malformation that primarily affects male fetuses;it is characterized by pulmonary hypoplasia, skeletal malformation, and kidney abnormalities. The pressure of the uterine wall due to oligohydramnios leads to an unusual facial appearance, abnormal limbs in abnormal positions, or contractures. The fetus generally dies soon after birth due to respiratory insufficiency. The baby was a live preterm male, born to a 30-year-old multigravida, out of a non-consanguineous marriage via cesarean section. There was no liquor at the time of delivery. The baby did not cry immediately after birth and required resuscitation, followed by mechanical ventilation. Multiple congenital anomalies suggestive of Potter’s syndrome were noted including facial features, flattened nose, low protruding ear, retrognathism, and epicanthal folds with unilateral atresia of the choana. Chest X-ray showed small volume lung fields suggestive of pulmonary hypoplasia, and we had on ultrasonography bilateral polycystic kidney disease on ultrasonography. At 42 hours of life, the baby developed tachypnea and severe chest retractions and died due to respiratory insufficiency. Our case highlights the importance of regular prenatal checks and examinations in each pregnancy, which helps to collect suspected cases and improve knowledge of this syndrome for better management.

Renal Vein Thrombosis Suggestive of Extramembranous Glomerulonephritis Associated with Sjögren’s Syndrome (Case Report)

Introduction: Glomerular damage during Gougerot-Sjgren syndrome is much rarer than interstitial damage, and is essentially extra-membranous and membrano-proliferative glomerulonephritis. Observation: We report the case of a 44-year-old woman with primary Sjgrens syndrome, confirmed by clinical dryness syndrome, positive anti-SSA and anti-SSB antibodies, and a salivary gland biopsy revealing grade 4 lymphocytic sialadenitis according to CHISHOLMs classification. Later, the patient developed nephrotic syndrome, along with hypertension. Renal function remained normal with a creatinine level of 9.3 mg/l, and hematuria was absent. Only antinuclear antibodies tested positive, while anti-PLA2R antibodies were negative. A renal biopsy was performed, which was complicated on the same day by hemodynamic instability with hematuria. Renal CT scan with contrast injection revealed a posterior perirenal hematoma without contrast extravasation. Additionally, bilateral renal vein thrombosis was incidentally discovered, suggesting extramembranous glomerulonephritis. The patients hemodynamic status stabilized after fluid resuscitation with isotonic saline solution (0.9%), without the need for blood transfusion. Renal biopsy confirmed extramembranous glomerulonephritis with interstitial fibrosis and minimal tubular atrophy. The initial etiological assessment was negative. The patient was started on oral corticosteroids, angiotensin-converting enzyme inhibitors, and therapeutic anticoagulation for renal vein thrombosis. The patients condition improved, with the disappearance of the syndrome and spontaneous regression of the hematoma. Discussion: The association of nephrotic syndrome and renal vein thrombosis primarily suggests glomerulopathy, in particular extra-membranous glomerulonephritis. Sjgrens syndrome can be associated with extra-membranous glomerulonephritis without being its direct cause. Like, it is possible that it is a cause of glomerulonephritis, essentially extra membranous and membrano-proliferative. Conclusion: Sjgrens syndrome is generally underestimated cause of glomerulonephritis, which should be considered in cases of extra-membranous glomerulonephritis.